NDLI: Prions and manganese: A maddening beast

Content Provider	Royal Society of Chemistry (RSC)
Author	Brown, David R.
Copyright Year	2011
Abstract	The prion protein is well known because of its association with prion diseases. These diseases, which include variant CJD, are unusual because they are neurodegenerative diseases that can be transferred between individuals experimentally. The prion protein is also widely known as a copper binding protein. The binding of copper to the prion protein is possibly necessary for its normal cellular function. The prion protein has also been suggested to bind other metals, and among these, manganese. Despite over ten years of research on manganese and prion disease, this interaction has often been dismissed or at best seen as a poor cousin to the involvement of copper. However, recent data has shown that manganese could stabilise prions in the environment and that chelation therapy specifically aimed at manganese can extend the life of animals with prion disease. This article reviews the evidence for a link between prions and manganese.
Starting Page	229
Ending Page	238
Page Count	10
File Format	HTM / HTML PDF
ISSN	17565901
Volume Number	3
Issue Number	3
Journal	Metallomics
DOI	10.1039/c0mt00047g
Language	English
Publisher	Royal Society of Chemistry
Access Restriction	Open
Subject Keyword	Prion Copper Chelation therapy Protein Manganese
Content Type	Text
Resource Type	Article
Subject	Chemistry Medicine Metals and Alloys Biochemistry Biomaterials Biophysics

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Prions and manganese: A maddening beast